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Ewing sarcoma

An Ewing sarcoma is the second most common malignant bone tumor in children and young adults. This form of cancer was first described in 1921 by James Ewing. An Ewing sarcoma can also occur in the so-called soft tissue (muscles and connective tissue). This bone tumor occurs annually in 1.5 per 1 million children under the age of 18. This means that every year 3-5 new children are discovered to have an Ewing sarcoma in the Netherlands. This bone tumor occurs in children and adults between 5 and 30 years of age with a peak between the ages of 10 and 15. An Ewing sarcoma is found more in boys than in girls (ratio 1.5:1) and more in the white race. In half of the cases, the tumor is found in the arms and legs, and in the other half in the torso bones.

Symptoms of this disease

The most significant symptoms are pain and swelling of the affected bone, resulting in restricted movement. In addition, a bone fracture may occur due to a lack of bone strength. Pain and possible nerve damage can occur during ingrowth in nerve tissue. In 15-35% of patients, metastases are found on diagnosis, mainly in the lungs, other bones and bone marrow.

How do we make the diagnosis?

A definitive diagnosis can only be made by obtaining tumor tissue using a biopsy. In addition, we take a bone photo and MRI scan of the location of the tumor. A CT scan of the lungs shows whether there are metastases here, a bone scan and a bone marrow puncture are required to assess whether there are metastases in the bones or bone marrow.

Treatment of Ewing sarcoma

The treatment of an Ewing sarcoma consists of a combination of chemotherapy, followed by surgery or radiation (radiotherapy). After the local treatment, there is often further chemotherapeutic after-treatment. For some children this is followed by therapy with a high dose of chemotherapy and a stem cell transplantation with the child's own stem cells. Sometimes the affected limb (arm or leg) needs to be amputated (see also osteosarcoma). If surgery is not required, radiation is a very important part of the treatment, in addition to chemotherapy.